Aflibercept for the Treatment of Macular Edema Secondary to Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis Syndrome.

We report a case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome in a patient whose cystoid macular edema (CME) was successfully treated with aflibercept and pan-retinal photocoagulation (PRP). A 56-year-old male was sent to our uveitis service for further evaluation after a fluorescein angiogram revealed symmetric retinal ischemia for 360 degrees in both eyes. A fundus examination revealed an aneurysm, neuroretinitis, and occlusive vasculitis, all consistent with a diagnosis of IRVAN syndrome. An optical coherence tomography examination revealed CME of the left eye. A chest X-ray revealed minimally prominent interstitial markings. The patient had a positive QuantiFERON-TB Gold test and was treated for tuberculosis with a one-year course of isoniazid and pyrimethamine. A further workup for other infectious and autoimmune etiologies was negative. The initial treatment consisted of bilateral PRP of the areas of peripheral ischemia, treatment for which was provided in a fragmented fashion over the course of seven months. Soon after the diagnosis, he received treatment with two intravitreal injections of aflibercept (2 mg/0.5 mL), one month apart, to the left eye. Subsequently, four months following the presentation, he developed CME in the right eye, which was treated with a single intravitreal injection of aflibercept (2 mg/0.5 mL). At his last follow-up visit, four years after the initial presentation, the patient remained asymptomatic with 20/20 visual acuity in both eyes and no evidence of CME recurrence. Our case suggests that aflibercept may serve as an adjuvant to the standard treatment with PRP, especially in cases that present with associated macular edema.

Adult Refsum Disease in Puerto Rico: A Case Report.

Patients with adult Refsum Disease (ARD) have retinitis pigmentosa and thus nyctalopia, anosmia, sensorineural deafness, polyneuropathy, and ataxia. Upon physical examination, patients with ARD have congenital short metacarpals, metatarsals, and cardiac arrhythmias. Manifestations due to the lack of phytanoyl-CoA hydroxylase in peroxisomes needed for alpha-oxidation of phytanic acid lead patients to accumulate phytanic acid in their body tissues. To our knowledge, no consensus for clinical diagnostic criteria for patients with ARD has been published. Our patient had nyctalopia, retinal findings, and visual field results compatible with retinitis pigmentosa. Additionally, the patient had decreased macular thickness and volume in both eyes, the findings being worse in the left eye. The patient had undergone hand surgery due to chronic pain in both hands, as well as his fourth and fifth metatarsal bones were shortened. Interestingly, audiology evaluation showed mild hearing loss in the right ear and mild to moderate hearing loss in the left ear. Inheritance patterns in patients with ARD have been described. Physical examination, phytanic acid evaluation, and genetic studies may all help reach an ARD diagnosis. This is the first report of adult Refsum disease in Puerto Rico.

Examining Global Crises: Extracting Insights From the COVID-19 Pandemic and Natural Disasters to Develop a Robust Emergency Diabetic Retinopathy Strategy for Puerto Rico.

In this critical analysis, we investigate the profound impact of natural disasters and pandemics on the care and adherence to treating diabetic retinopathy, a severe complication of diabetes requiring continuous monitoring and treatment to prevent vision loss. Our study also sheds light on the social and economic context of Puerto Rico, emphasizing recent emergency events that have exacerbated existing public health challenges. Through a comprehensive review of relevant literature from PubMed, Google Scholar, and the George Washington University Himmelfarb Health Sciences Library database, we identified 31 pertinent articles out of 45 evaluated, focusing on the effects of these crises on healthcare delivery, diabetic retinopathy screening, and treatment. The evidence strongly indicates that during such emergencies, barriers to healthcare escalate, leading to significant treatment delays and a reduction in diabetic retinopathy screening and diagnosis, ultimately resulting in deteriorated visual outcomes. Thus, our review underscores the urgent need for the development of effective emergency plans tailored specifically to diabetic retinopathy, particularly in Puerto Rico, where diabetes prevalence and its complications are notably higher. Such plans should not only incorporate established emergency measures but also harness emerging technological advances in the field of ophthalmology to ensure optimal preparedness for future pandemics and natural disasters.

Bilateral Acute Retinal Necrosis Treated With Antivirals and Corticosteroids.

We report on the case of a Hispanic male with bilateral acute retinal necrosis (BARN), whose disease was managed with intravenous acyclovir, topical prednisolone, topical atropine, intravitreal ganciclovir, oral valacyclovir, and oral prednisone. The oral corticosteroid was added to his treatment regimen eight days after his initial presentation. The 55-year-old patient presented with a two-week history of bilateral blurred vision. His medical history was pertinent for remote varicella and herpes zoster (the latter limited to the forehead). His best-corrected visual acuity (BCVA) was counting fingers in both eyes (OU). His examination was remarkable in OU for grade 3+ cells, pharmacologically dilated pupils, and grade 2+ vitreous cells. The patient's fundus was remarkable in OU for optic-nerve swelling, vitreous condensation, ghost vessels, and retinitis patches. Given the clinical and ancillary testing results, an assessment of BARN was made. The patient received acyclovir treatment initially, and systemic steroids were introduced eight days later. He ultimately experienced significant clinical improvement.

Systemic Disease Associations in a Cohort of Hispanic Patients with Scleritis.

(1) Purpose: A patient with scleritis may have an associated systemic disease, which is often autoimmunological and seldom infectious in origin. The data regarding such associations in Hispanic populations are scarce. Therefore, we evaluated the clinical characteristics and systemic-disease associations of a cohort of Hispanic patients with scleritis. (2) Methods: A retrospective review of the medical records (January 1990-July 2021) of two private uveitis practices in Puerto Rico was performed. Clinical characteristics and systemic-disease associations observed either at presentation or diagnosed as a consequence of the initial workup were recorded. (3) Results: A total of 178 eyes of 141 patients diagnosed with scleritis were identified. An associated autoimmune disease was present in 33.3% of the patients (rheumatoid arthritis, 22.7%; Sjögren's syndrome, 3.5%; relapsing polychondritis, 2.8%; sarcoidosis, 1.4%; systemic lupus erythematosus, 1.4%; and systemic vasculitis, 0.7%). An associated infectious disease was present in 5.7% of the patients (2.13%, syphilis; 1.41%, herpes simplex; 1.14%, herpes zoster; and 0.71%, Lyme disease). One patient had all-trans retinoic-acid-associated scleritis. Statistical analysis revealed that patients with nodular anterior scleritis were less likely to have an associated immune-mediated disease (OR: 0.21; p = 0.011). (4) Conclusion: Rheumatoid arthritis was the most common systemic autoimmune disease association, while syphilis was the most common infectious disease associated with scleritis patients. Our study suggests that patients with nodular scleritis have a lower risk of having an associated immune-mediated disease.